HD is a genetically caused brain disorder that causes uncontrollable bodily movements and robs people's ability to walk, talk, eat, and think. The final result is a slow, ugly death. Children of parents with HD have a 50-50 chance of inheriting the disease. There is no cure or treatment.

HD Blogs and Individuals

Monday, May 25, 2009

People respond to a diagnosis of Huntington’s disease in many ways, ranging from complete denial to optimism about the research for a cure. Indeed, in order to cope, a person with HD or at risk for the disease can experience the entire range of feelings within his or her own life – perhaps in just one day.

In the last decade, I myself have gone through dark moments of thinking that my symptoms had started and also felt near euphoria after learning of potential breakthroughs towards an effective treatment. I tested positive for Huntington’s in 1999, and my mother died of the disease in 2006 at age 68.

But some reactions, falling into a category all by themselves, stir up the deepest of emotions.

Changing her identity

I got that feeling this past week when I read the following anonymous comment on my previous blog posting (click here to read it). It reads:

“Thank you, Gene. I am new to this forum and my connection with HD is an in-law who has begun showing signs. I have wept much since she first confided in me (only five people in the world know as she changed her identity and left home long ago to escape the horror). She is the bravest person I have ever met. I do not know how you live with the time bomb, and I pray for her continually to know a measure of peace. I am adding you to my prayer list as well.”

Reading about this person’s decision to change her identity and move away sank my heart. I felt deep sadness and empathy for this person. I know nothing else about her, but I believe I know exactly what she is feeling.

Like an injured animal that knows it will not survive, she has gone off into the wilderness to die.

She reminded me of Phebe Hedges, a 40-year-old woman with HD who walked into the sea in an act of suicide, as described in Alice Wexler’s book about Huntington’s, The Woman Who Walked into the Sea (click here to read about the book).

The instinct to protect

The woman who fled acted on instinct, seeking to remove herself from all of the social horrors often experienced by the affected and their families, including denial, stigma, and discrimination.

Her instinct also led her to preserve those around her from witnessing the terrible symptoms of HD such as the inability to walk, talk, think, swallow, and remember. As her brain dies and her humanity disappears, their lives will go on in relative tranquility.

I remember visiting my mother for the first time after her diagnosis. I had not seen her since her and my father’s trip to visit my wife and me in California. I had urged my father to seek out a specialist to get to the root of my mother’s unexplained strange behavior. Now, speaking about her diagnosis, she appeared very sad and depressed. She was upset because she could not stop saying “yep.” Her self-control was disappearing. She appeared helpless and bewildered.

Looking back on that moment, I think she must have had those same deep, instinctual feelings about her impending decline.

Thoughts of suicide

The story of the woman who fled evoked my own thoughts about suicide. In the first years after my positive test for Huntington’s disease, I told my wife and some close friends that I would prefer to die with my health relatively intact than to be forced to watch myself lose my ability to write, work, and enjoy my family.

I fantasized about organizing a farewell ceremony at which I would announce my decision to my friends and family and then privately take my leave of the world. This, I thought, would be better than flailing about lonely and mindlessly while being tied down to a chair or confined to a room in a nursing home, the fate of some HD patients.

I am hoping that the memory of those dark thoughts has come alive again only for a short time.

I will need complete focus as yet another stage looms in my personal battle against HD: the need to become more public in my activism.

We must speak out

I would never criticize the woman who fled, for I saw her pain in my mother’s face and have denied my own at-risk status in numerous ways. As my readers know, I use a pseudonym on this blog – my own self-protection because of the horrors of HD. And if I develop full-blown, highly debilitating symptoms, suicide might once again look like the only way out.

But at this juncture I want to go down fighting. And I have come to believe that, despite whatever happens to me because of HD, my life can still have deep meaning and significance.

As one caregiver stated angrily at a Huntington’s disease support group meeting that I attended last month, nobody will notice – or help – affected individuals and their families unless we speak out and let the world know about the enormous challenges posed by HD.

A dream about going public

Two nights ago, after I had started writing this entry, I dreamt very clearly about Huntington’s. In the dream my co-workers – who in reality and in the dream do not know about my situation because of my fear of discrimination, and of losing my job or not getting a better one – are speaking enthusiastically about a new and deeply worthy cause: HDSA. They are planning to attend an exciting fundraising event that the organization is putting on in a few days (click here to read more).

Near the end of the dream I decide to reveal my story to this warm and understanding group of people.

Like the woman who fled, I cannot escape the horrors of Huntington’s disease. But, unlike the animal that dies alone in the wilderness, I can tap into a community for help – a community of HD families, activists, and supporters who are working to find treatments and a cure.

As I often write to others in the community, together we can beat this disease.

Saturday, May 16, 2009

Knowing that the first, subtle, and practically imperceptible symptoms of Huntington’s disease are most likely already at work in my brain and body, I have become hyper-conscious about the brevity of life.

We are all in a race to death, but people at risk for life-shortening diseases know that their time to the finish line is painfully fast and troubled. In my case these feelings erupted to the surface of my being as I saw my mother in the devastating final stages of Huntington’s disease in 2005 and early 2006 (click here to read more). They intensified as I dealt with deep grief about her death and my future as an at-risk individual after attending her funeral in February 2006.

Since then I have increasingly thought of the acronym “HD” in a very different light. For me it stands not only for “Huntington’s disease,” but also for the fact that I am “hurtling towards death.”

I’ve been a workaholic and a perfectionist since childhood. I feel a fanatical urge to “get everything done” before I start having perceptible, debilitating symptoms. And lately the need to build a prominent legacy has consumed me.

Striving for efficiency

On the professional front, it’s not been enough for me to publish several respected books in my field. I strive for ways to liberate my creative activities in the same way that led Isaac Asimov to write hundreds of books.

At home and on the computer I am constantly responding to and/or saving the many e-mails I receive each day from four different accounts, and I am organizing our family papers into files and file boxes.

On the family front, I’ve been pushing my eight-year-old daughter to practice her piano as we prepare for our second annual piano-guitar duet next month. I pick her up from school three days a week and am in charge of the home for many hours, as my wife also works full time. I regularly have to stop myself from turning our relationship into yet another opportunity to value efficiency above all in my life.

My relationship with my wife has increasingly revolved around raising our daughter, as neither of us has relatives nearby to help with her care or to nurture family ties. As she finishes the third grade, planning for a possible transfer to private school – with her college education in mind – has taken on greater urgency.

We’ve also been spending lots of time and money remodeling our home, and, as I wrote recently (click here to read the entry), we’ve now taken on the building of a swimming pool. The pool will be an antidote to the hectic pace of our lives, but for now its construction is a new source of stress as we negotiate with contractors, watch our back yard turn into a massive hole, and put up with the din of machinery.

A decade of activism

Last month I completed eleven years as an activist for the local chapter of the Huntington’s Disease Society of America (HDSA). For seven of those years I wrote, edited, and published the chapter newsletter and participated in a plethora of other activities, from taking notes at board meetings and folding the newsletters to raising money and organizing meetings of HD advocates with California’s stem-cell oversight board in the quest for funding HD research.

My latest project was to oversee the overhaul of the chapter web site that I inaugurated in 2002. With limited funds and time, it was a time-consuming but rewarding task. This blog will now be published on the site too, and I will manage and edit the site and produce articles for it (click here to visit the site).

All of this frenetic activity has come to a head as I worry about the health of my father, a Huntington’s disease warrior who cared for my mother for 15 years and who has declined rapidly in the past few months (click here to read that entry).

HD people: like anybody else

Two weekends ago someone blind-sided my car, sending me to the hospital briefly and causing $3,000 in damage to my vehicle. I escaped with just a sore neck, but it was the first time I was involved in an accident. The shock left me emotionally paralyzed for the better part of a week, and the hassle of dealing with insurance companies, getting a rental car, and finding a repair shop threw off my focus.

“This can’t happen to me!” I angrily told myself. “I have HD to worry about! I have an agenda to meet and a mission to fulfill!” I was deeply upset at being knocked off balance and losing precious time. As a result, I couldn’t work on this blog for a couple weeks.

I had a hard time understanding that people at risk for HD are just like anybody else. Then I remembered an at-risk friend who got breast cancer just before she started having HD symptoms. Being at risk for HD does not make me immune from the rest of life’s challenges.

Unable to dream of the future

With the help of a psychotherapist I struggle to keep juggling the many balls of my life as I face the inevitability of my symptoms. The pace grows ever faster, and I am frustrated that I don’t have another six hours each day.

I sometimes fantasize about how calm things would be if I were not hurtling ahead into Huntington’s disease. I could perhaps follow the natural rhythm of life, slowing down as I proceed into my fifties and look forward to retirement, my daughter’s college, and her wedding in my sixties.

But that’s where my imagination stops, because my mother started showing signs of HD in her late forties and had full-blown HD by her mid-fifties. The last ten years of her life she hardly spoke and eventually ended up in a wheelchair. By age 68, when she died, she was a mere shadow of herself.

She never really got to know her granddaughter. And now, because of my fear of following in my mother’s footsteps, I can’t even dream about the day my daughter will bring her own child into the world.

So instead of relaxing, I often accelerate my work with HDSA and my other projects. Every day I try to squeeze in as much life as possible.

The fight against HD often feels long and weary. My other passions and manias often help me escape the thought of HD. But they have often left me exhausted.

So, as I write this today, I start to focus on an important question: what should my most important legacy be? But before I even begin to answer this question, I need to catch my breath.